Accumulation of toxic amounts of prelamin A, either due to LMNA mutations, or due to mutation of the prelamin A endoprotease ZMPSTE24, which catalyzes protein maturation, is the molecular basis of Hutchinson-Gilford progeria syndrome (HGPS), Mandibuloacral dysplasia with accelerated ageing and type A (MADA, OMIM #248370) or type B lipodystrophy (MADB) and Restrictive Dermopathy (RD, OMIM #275210), a severe developmental disorder [6, 7] [8, 9]. The gene discussed is ZMPSTE24; the disease is Hutchinson-Gilford progeria syndrome.